Bilateral Bell Palsy as a Presenting Sign of Preeclampsia Alison Vogell, MD, Rupsa C. Boelig, MD, Joanna Skora, BS, and Jason K. Baxter, MD BACKGROUND: Bell palsy is a facial nerve neuropathy that is a rare disorder but occurs at higher frequency in pregnancy. Almost 30% of cases are associated with preeclampsia or gestational hypertension. Bilateral Bell palsy occurs in only 0.3%–2.0% of cases of facial paralysis, has a poorer prognosis for recovery, and may be associated with a systemic disorder. CASE: We describe a case of a 24-year-old primigravid woman with a twin gestation at 35 weeks diagnosed initially with bilateral facial palsy and subsequently with preeclampsia. She then developed partial hemolysis, elevated liver enzymes, and low platelet count syndrome, prompting the diagnosis of severe preeclampsia, and was delivered.

including hyperreflexia.1 Bell palsy is a rare facial neuropathy that not only is more common in pregnant women than in nonpregnant women but also has been associated with hypertensive disorders of pregnancy.2–4 One study found that almost 30% of women with Bell palsy had preeclampsia or gestational hypertension, nearly five times the expected rate.2 Bell palsy has also been associated specifically with severe preeclampsia.3 A PubMed search from 1978 to 2013 of “bilateral facial palsy and pregnancy” revealed only five case report series on bilateral facial paralysis in pregnancy, none of which involved a hypertensive disorder. Here we report the case of a 24-year-old with a twin gestation at 35 weeks who presented with bilateral facial paralysis. She subsequently developed severe preeclampsia and was delivered.

CASE

© 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14

A 24-year-old woman, gravida 1 para 0, at 35 2/7 weeks of gestation with a dichorionic twin pregnancy presented to a community emergency department with acute onset of unilateral facial palsy. Two days before presentation, the patient noticed left retroauricular and mastoid pain followed by weakness and numbness of her left lip that then progressed to involve the entire left side of her face and loss of taste. Her blood pressure and other vital signs were normal. An ultrasound examination demonstrated a dichorionic twin gestation with fetuses A and B measuring appropriately. The patient was diagnosed with unilateral Bell palsy, treated with acyclovir and prednisone, and discharged home. The following day, she developed weakness and numbness of the contralateral face and returned to the emergency department. She denied any fevers or chills, recent illness, diploplia, loss in vision, changes in hearing, paresthesias, or rashes. Her white count was 11.23109/L, and complete blood count and comprehensive metabolic panel were otherwise normal. The patient’s antenatal course was complicated by pyelonephritis and nephrolithiasis that were treated 1 month before presentation. Her medical history was significant only for mononucleosis, salmonella, and shingles infections as an adolescent. The patient was transferred to our institution for further evaluation. On admission, her blood pressure range was 137–141/82–85 mm Hg. She denied any headaches, changes in vision, right upper quadrant pain, shortness of breath, nausea, or emesis. On physical examination, the patient’s pupils were equally round and reactive and her extraocular movements intact. She was unable to completely close her eyes, wrinkle her forehead, purse her lips, or close her lips tightly. There was no nuchal rigidity, her strength was intact throughout, and her deep tendon reflexes were 3+ in her upper extremities and 2+ in her lower extremities with no clonus. She had no rash or vesicular eruption. Initial laboratory findings were notable for 3+ protein on urine

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CONCLUSION: Bilateral facial palsy is a rare entity in pregnancy that may be the first sign of preeclampsia and suggests increased severity of disease, warranting close monitoring. (Obstet Gynecol 2014;124:459–61) DOI: 10.1097/AOG.0000000000000221

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reeclampsia is a hypertensive disorder of pregnancy with myriad manifestations. This disorder affects 5–8% of pregnancies.1 The diagnosis of preeclampsia is made when maternal blood pressure is greater than 140/90 mm Hg with proteinuria greater than 300 mg in a 24-hour period. Severe preeclampsia is defined by the presence of at least one other criteria, including blood pressure greater than 160/100, persistent headache or visual changes, or hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Patients with preeclampsia may also have peripheral edema and neurologic aberrations

From the Department of Obstetrics and Gynecology, Jefferson Medical College, and the Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania. Corresponding author: Rupsa C. Boelig, MD, 833 Chestnut Street, Suite M-132, Philadelphia, PA 19107; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest.

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analysis, platelet count of 128,000/microliter, hemoglobin of 10.9 g/dL, and white count of 103/ml. Liver and kidney function tests were within normal limits. Cerebral spinal fluid demonstrated normal levels of protein, glucose, white blood cells, total cell count, and a negative gram stain. Brain magnetic resonance imaging showed no pathology. The patient was started on eye drops for lubrication. Given the leukocytosis and concern for an inflammatory process, she was continued on acyclovir and a prednisone taper. On hospital day 3, the patient’s blood pressures continued to be elevated, and she was found to have 1,872 mg of protein in a 24-hour urine collection. Her platelet count declined to 99,000/microliter. Given her declining platelets with concern for HELLP syndrome, she was diagnosed with severe preeclampsia, started on magnesium sulfate for seizure prophylaxis, and delivered via cesarean for breech presentation of fetus A. The procedure was uncomplicated; both neonates weighed appropriately and did well. Postpartum, the patient’s blood pressure and platelets normalized. However, her facial palsy improved only slightly. Her neurologic workup, which included testing of blood and cerebral spinal fluid for a variety of infectious etiologies including Lyme disease, Campylobacter jejuni, Listeria monocytogenes, hepatitis, human immunodeficiency virus (HIV), toxoplasmosis, and syphilis and screening for autoimmune diseases including Sjo¨ gren syndrome, systemic lupus erythematosus, sarcoidosis, and multiple sclerosis, was negative. Although this patient had a history of herpes zoster, she had no evidence of reemergence. Given the difficulty in identifying a source, the patient was continued on acyclovir and prednisone to complete a 7-day course, but it was concluded that the etiology was likely related to preeclampsia. By 6 weeks postpartum, the patient’s symptoms improved dramatically. She had only a slight left droop of her mouth and the inability to completely elevate her left eyebrow. Neurologic assessment recommended no further workup or treatment.

COMMENT The incidence of Bell palsy in pregnant women (45/100,000 births) is higher than the incidence in nonpregnant women (17/100,000). 5 It presents almost exclusively in the third trimester or postpartum and may be caused by increased peripheral edema leading to compression of the facial nerve, immunosuppression predisposing to viral infection or reactivation, or changes in levels of estrogen, progesterone, and cortisol. 5,6 There have been several case reports in the literature citing examples of Bell palsy presenting in the third trimester with a hypertensive disorder of pregnancy.4,5 Shmorgun et al 2 identified a series of 41 patients presenting with Bell palsy during pregnancy, 29% of whom were diagnosed with preeclampsia or gestational

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hypertension. Of note, these reports are of unilateral facial palsy. Physiologic changes in the third trimester of pregnancy that predispose to Bell palsy are exacerbated by preeclampsia. The association of Bell palsy with preeclampsia has been attributed to peripheral edema from preeclampsia leading to compression of the facial nerve and subsequent paralysis, and the hypercoagulable state in preeclampsia leading to thrombosis in the vasa nervorum of the facial nerve, resulting in decreased blood supply to the facial nerve and paralysis.2 Additionally, preeclampsia is associated with alterations in immune response that may also predispose to Bell palsy.1 That multifetal pregnancies are at higher risk for preeclampsia suggests that they may be at higher risk for Bell palsy as well; in fact, in the series by Shmorgun et al,2 7% of patients with Bell palsy were twin gestations. Bilateral palsy occurs in only 0.3%–2.0% of facial paralysis cases.7 It is often associated with a systematic disease and is a poor prognostic factor, with increased risk of permanent damage.6–8 The differential diagnosis for simultaneous bilateral facial paralysis includes Lyme disease, syphilis, Guillain-Barré syndrome, infectious mononucleosis, postinfluenza syndrome, HIV infection, herpetic infection, and sarcoidosis.7,8 Management should include neurologic consultation— and ophthalmologic consultation in the setting of ocular symptoms—treatment with acyclovir for suspected herpetic infection, and treatment with prednisone, although its use and efficacy are controversial.7,8 Given the poorer prognosis of bilateral palsy, induction of labor has been suggested for those near term.8 We have reported a case of a dichorionic twin gestation with acute idiopathic bilateral facial nerve paralysis, a unique initial presentation of severe preeclampsia. This case adds to the current literature examining the relationship between facial palsy and preeclampsia and goes further to suggest that the rare case of bilateral facial palsy may indicate increased severity of preeclampsia. The presentation of bilateral facial palsy warrants close monitoring for the development of preeclampsia and consideration of an induction of labor for those near term in an attempt to improve the prognosis for recovery from the paralysis. REFERENCES 1. Diagnosis and management of preeclampsia and eclampsia. ACOG Practice Bulletin No. 33. American College of Obstetricians and Gynecologists. Obstet Gynecol 2002;99:159–67. 2. Shmorgun D, Chan WS, Ray JG. Association between Bell’s palsy in pregnancy and pre-eclampsia. Q J M 2002;95:359–62.

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3. Katz A, Sergienko R, Dior U, Witzniter A, Kaplan DM, Sheiner E. Bell’s palsy during pregnancy: is it associated with adverse perinatal outcome? Laryngoscope 2011;121:1395–8.

6. Cohen Y, Lavie O, Granovsky-Grisaru S, Aboulafia Y, Diamant YZ. Bell palsy complicating pregnancy: a review. Obstet Gynecol Surv 2000;55:184–8.

4. Shapiro JL, Yudin MH, Ray JG. Bell’s palsy and tinnitus during pregnancy: predictors of pre-eclampsia? Three cases and a detailed review of the literature. Acta Otolaryngol 1999;119:647–51.

7. Jain V, Deshmukh A, Gollomp S. Bilateral facial paralysis: case presentation and discussion of differential diagnosis. J Gen Intern Med 2006;21:C7–10.

5. Hilsinger RL Jr, Adour KK, Doty HE. Idiopathic facial paralysis, pregnancy, and the menstrual cycle. Ann Otol Rhinol Laryngol 1975;84(4 pt 1):433–42.

8. Kovo M, Sagi Y, Lampl Y, Golan A. Simultaneous bilateral Bell’s palsy during pregnancy. J Matern Fetal Neonatal Med 2009;22:1211–3.

Multiple Myeloma Presenting as Hypercalcemic Pancreatitis During Pregnancy Jennifer McIntosh, DO, MS, Jacob Lauer, MD, Ravindu Gunatilake, MD, and Eric Knudtson, MD BACKGROUND: Multiple myeloma is typically a disease found in older women and is a rare diagnosis in pregnancy. CASE: A 22-year-old woman, gravida 1 para 0, at 32 3/7 weeks of gestation presented with nausea, vomiting, and rib and back pain. She was hypertensive, anemic, thrombocytopenic, and in acute renal insufficiency, with hypercalcemia and laboratory parameters indicative of pancreatitis. She was admitted to the obstetric intensive care unit with working diagnoses of preeclampsia, pancreatitis, nephrolithiasis, and renal insufficiency. She ultimately was delivered because of declining clinical status, and multiple myeloma eventually was diagnosed as the underlying cause of her myriad of problems. CONCLUSION: Multiple myeloma is unusual during pregnancy. However, in patients with significant and unexplained hypercalcemia, malignancy should remain high on the differential diagnosis. (Obstet Gynecol 2014;124:461–3) DOI: 10.1097/AOG.0000000000000361

From the Department of Obstetrics and Gynecology, University of Oklahoma, Oklahoma City, Oklahoma. Corresponding author: Dr. Eric Knudtson, MD, Department of Obstetrics and Gynecology, 920 Stanton L Young Blvd. WP 2410, Oklahoma City, OK 73104; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14

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ultiple myeloma is a hematologic malignancy with a median age at diagnosis of 65 years. It accounts for 1% of all cancers and 10% of all hematologic malignancies. Only 2% of multiple myeloma cases are diagnosed in those younger than 40 years of age. The typical clinical presentation of the disease includes anemia, bone pain, elevated creatinine, hypercalcemia, and weight loss.1 The diagnosis of multiple myeloma is suspected in the presence of a monoclonal protein in the serum or urine that demonstrates an increased kappa-to-lambda ratio. Bone marrow biopsy results demonstrate significant nucleated or malignant-appearing plasma cells, with immunohistochemistry demonstrating kappa light chain restriction.2 Treatment involves chemotherapy followed by autologous stem cell transplantation. Prognosis is typically poor, with 25% survival at 5 years and 9% survival at 10 years.3 Multiple myeloma in pregnancy is rare. We present a case of a gravid woman who presented with severe hypercalcemic pancreatitis that resulted in a diagnosis of multiple myeloma.

CASE A 22-year-old woman, gravida 1 para 0, at 32 3/7 weeks of gestation presented to the obstetric triage unit at the University of Oklahoma with symptoms of nausea, vomiting, and rib and back pain. She described epigastric pain but denied headaches or visual disturbances. She reported fetal movement and denied symptoms of labor. Her medical history was noncontributory, and aside from mild anemia at her first prenatal visit, her pregnancy was otherwise unremarkable. On examination the patient was hypertensive, with initial blood pressure of 162/95 mm Hg. Laboratory test results demonstrated significant anemia, thrombocytopenia, and acute renal insufficiency. Calcium, amylase, and lipase levels were aberrant at 20.0 mg/dL (corrected), 2,439 units/L, and 1,288 units/L, respectively. Additionally, she had an elevated protein-to-creatinine ratio of 1.8. An abdominal ultrasonogram was performed and revealed a diffusely enlarged and echogenic pancreas without evidence of necrosis, a nonobstructing nephrolithiasis, hepatomegaly without focal mass lesion, and a normal gallbladder.

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